Ethical Implications of Cleft Lip and Palate Repair in Patients with Trisomy 13 and Trisomy 18.

IF 1.2 4区 医学 Q3 DENTISTRY, ORAL SURGERY & MEDICINE Cleft Palate-Craniofacial Journal Pub Date : 2024-08-01 Epub Date: 2023-03-21 DOI:10.1177/10556656231163722
Richard Appel, Andrew E Grush, Raghave M Upadhyaya, David G Mann, Edward P Buchanan
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Abstract

Background: Children born with Trisomy 13 or 18 (T13/18) often have multiple congenital anomalies, many of which drastically shorten their lifespan. Among these defects are cleft lip and palate, the repair of which presents an ethical dilemma to the surgeon given the underlying comorbidities associated with T13/18. The authors present an ethical discussion and institutional experience in navigating this dilemma.

Methods: The authors analyzed existing literature on T13 and T18 surgery and mortality. A retrospective study over ten years was also conducted to identify pediatric patients who underwent surgical correction of cleft lip and/or palate secondary to a confirmed diagnosis of T13/18. The authors identified two patients and examined their treatment course.

Results: The authors' review of literature coupled with their institution's experience builds on the published successes of correcting cleft lip and palate in the setting of T13/18. It was found that both patients identified in the case series underwent successful correction with no surgical complications.

Conclusion: A careful balance must be struck between improved quality of life, benefits of treatment, and risks of surgery in children with T13/T18. Careful consideration should be given to the medical status of these complex patients. If the remaining medical comorbidities are well managed and under control, there is an ethical precedent for performing cleft lip and palate surgeries on these children. A diagnosis of T13/T18 alone is not enough to disqualify patients from cleft lip/palate surgery.

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13 三体综合征和 18 三体综合征患者唇腭裂修复的伦理意义。
背景:患有 13 或 18 三体综合征(T13/18)的儿童通常会有多种先天性畸形,其中许多会大大缩短他们的寿命。这些缺陷中包括唇腭裂,鉴于 T13/18 三体综合征的潜在并发症,修复唇腭裂给外科医生带来了伦理难题。作者介绍了应对这一困境的伦理讨论和机构经验:作者分析了有关 T13 和 T18 手术及死亡率的现有文献。作者还进行了一项长达十年的回顾性研究,以确定因确诊为 T13/18 而接受唇裂和/或腭裂手术矫正的儿童患者。作者确定了两名患者,并检查了他们的治疗过程:结果:作者对文献的回顾以及他们所在医疗机构的经验是在已发表的 T13/18 唇腭裂矫正成功案例的基础上发展而来的。结果:作者的文献综述及其所在机构的经验,是在已发表的 T13/18 兔唇和腭裂成功矫正案例的基础上总结出来的:结论:对于患有 T13/T18 的儿童,必须在改善生活质量、治疗效果和手术风险之间取得谨慎的平衡。应仔细考虑这些复杂患者的医疗状况。如果其余的并发症得到很好的管理和控制,那么为这些儿童实施唇腭裂手术是符合伦理的先例。仅凭 T13/T18 诊断并不足以取消患者接受唇腭裂手术的资格。
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来源期刊
CiteScore
2.70
自引率
36.40%
发文量
215
期刊介绍: The Cleft Palate-Craniofacial Journal (CPCJ) is the premiere peer-reviewed, interdisciplinary, international journal dedicated to current research on etiology, prevention, diagnosis, and treatment in all areas pertaining to craniofacial anomalies. CPCJ reports on basic science and clinical research aimed at better elucidating the pathogenesis, pathology, and optimal methods of treatment of cleft and craniofacial anomalies. The journal strives to foster communication and cooperation among professionals from all specialties.
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